Estimation of skeletal muscle energy metabolism in Machado-Joseph disease using 31P-MR spectroscopy

✍ Scribed by Ichiro Yabe; Khin K. Tha; Takashi Yokota; Kazunori Sato; Hiroyuki Soma; Asako Takei; Satoshi Terae; Koichi Okita; Hidenao Sasaki

• Publisher: John Wiley and Sons
• Year: 2010
• Tongue: English
• Weight: 840 KB
• Volume: 26
• Category: Article
• ISSN: 0885-3185
• DOI: 10.1002/mds.23335

✦ Synopsis

Abstract

The aim of this study was to determine if muscle energy metabolism, as measured by ^31^P‐magnetic resonance spectroscopy (MRS), is a metabolic marker for the efficacy of treatment of Machado‐Joseph disease (MJD). We obtained ^31^P‐MRS in the calf muscle of 8 male patients with MJD and 11 healthy men before, during, and after a 4 minute plantar flexion exercise in a supine position. The data showed that there was a significant difference between the groups in terms of the PCr/(Pi + PCr) ratio at rest (P = 0.03) and the maximum rate of mitochondrial ATP production (V~max~) (P < 0.01). In addition, V~max~ was inversely correlated with the scale for the assessment and rating of ataxia score (r = −0.34, P = 0.04). The MJD group also showed a reduction in V~max~ over the course of 2 years (P < 0.05). These data suggest that this noninvasive measurement of muscle energy metabolism may represent a surrogate marker for MJD. © 2010 Movement Disorder Society